Wernekink Commissure Syndrome With Hallucinations and Involuntary Groping:Report of One Case / 中国医学科学院学报

Wernekink commissure syndrome is a rare midbrain syndrome with bilateral cerebellar dysfunction,eye movement disorder,and palatal myoclonus.Few cases of this syndrome have been reported in China,let alone those combined with hallucinations and involuntary groping.This paper reports the diagnosis and treatment of a case of Wernekink commissure syndrome with hallucinations and involuntary groping,aiming to enrich the knowledge about this disease for clinicians.

Síndrome de flutter ocular e ataxia de tronco pós-infecciosa / The post-infectious ocular flutter syndrome and truncal ataxia

OBJETIVO: Descrever um quadro clínico subagudo pós-infeccioso caracterizado por ataxia de tronco e flutter ocular. RELATO DE CASO: Um homem de 37 anos previamente hígido, pouco após um quadro sistêmico inespecífico que se resolveu espontaneamente, deu início a movimentos involuntários hipercinéticos dos olhos, da cabeça e desequilíbrio importante. Os sintomas evoluíram em poucos dias. Ao exame, apresentava ataxia de tronco e presença de flutter ocular. O líquor mostrou pleocitose discreta. Ressonância magnética (RM) de crânio e exames laboratoriais normais. Houve melhora espontânea e total dos sintomas em cerca de três semanas. CONCLUSÃO: A síndrome de flutter ocular e ataxia de tronco é rara e é destacado a benignidade do quadro.

OBJECTIVE: To describe a post-infectious subacute clinical picture characterized by truncal ataxia and ocular flutter. CASE REPORT: A healthy 37-year-old man, a few days after spontaneously resolved nonspecific systemic disease, initiated involuntary hyperkinetic movements of the eyes, head, and major imbalance. The symptoms progressed within a few days. He presented ataxia of the trunk and presence of ocular flutter. CSF showed pleocytosis. Magnetic resonance of the skull and laboratory tests were normal. There was spontaneous and total improvement of symptoms in about three weeks. CONCLUSION: The ocular flutter syndrome and truncal ataxia is rare and the benignity of the condition is highlighted.

Esporotricose ocular: manifestações atípicas / Ocular sporotrichosis: atypical manifestations

Resumo A esporotricose humana e animal é uma infecção subaguda a crônica causada pelo fungo dimórfico Sporothrix schenckii. A esporotricose ocular tem ganhado destaque em função da epidemia de esporotricose urbana enfrentada pelo estado do Rio de Janeiro na última década e se apresenta classicamente como conjuntivite granulomatosa, mas formas atípicas podem ocorrer. Este artigo tem por objetivo relatar 2 casos atípicos de esporotricose ocular em pacientes imunocompetentes, ambos apresentando quadro clínico compatível com a síndrome oculoglandular de Parinaud associada à dacriocistite em um caso e presumivelmente à coroidite no outro caso.

Abstract Human and animal sporotrichosis is an infection caused by the dimorphic fungus Sporothrix schenckii, which is classified from subacute to chronic. Ocular sporotrichosis has been highlighted due to the epidemic of urban sporotrichosis faced by the state of Rio de Janeiro in the last decade and presents classically as granulomatous conjunctivitis, but atypical forms may occur. This article aims to report two atypical cases of ocular sporotrichosis in immunocompetent patients, both presenting a clinical picture compatible with Parinaud oculoglandular syndrome associated with dacryocystitis in one case and presumably to choroiditis in the other case.

Regeneración aberrante del III nervio craneal por aneurisma cerebral bilateral. Reporte de caso / Aberrant regeneration of the III cranial nerve due to bilateral cerebral aneurism

RESUMEN Los trastornos de la motilidad ocular constituyen motivo de consulta periódica en Oftalmología. La regeneración aberrante, trastorno muy poco reportado, es considerada la sincinesia oculomotora de mayor invalidez y complejidad. Diversas condiciones neuroftalmológicas están implicadas en la etiopatogenia de la enfermedad, la mayoría de las cuales puede ocasionar la muerte. El manejo de los síntomas y signos provocados por paradójicos movimientos oculares conjugados es difícil. Se reportó un caso con remisión tardía a neuroftalmología por diagnóstico inicial y evolución desfavorable. La historia psicofísica arrojó diagnóstico definitivo de regeneración aberrante del III nervio craneal secundario, a aneurisma cerebral de la carótida interna bilateral, agravado por reanastomosis quirúrgica. Una rigurosa, obligatoria e impostergable historia neuroftalmológica, se impone ante toda parálisis del III nervio craneal para brindar un diagnóstico etiológico preciso y de esta forma proteger la vida.

ABSTRACT The disturbances in ocular motility are the cause of periodical consultation in Ophthalmology. The aberrant regeneration, a scarcely reported disturbance, is considered the oculomotor synkinesis of highest disability and complexness. Several neuro-ophthalmologic conditions are implicated in the disease ethiopathogeny, and most of them could lead to death. The management of the symptoms and signs caused by paradoxical conjugated ocular movements is difficult. A case is reported of late remission to Neuro-ophthalmology due to unfavorable diagnosis and evolution. The psycho-physical history led to a definitive diagnosis of aberrant regeneration of the III secondary cranial nerve, to cerebral aneurism of the bilateral internal carotid, worsened by surgical re-anastomosis. In front of any paralysis of the III cranial nerve, it is necessary a rigorous, obligatory and immediate neuro-ophthalmological history to arrive to a precise etiological diagnosis, protecting life in that way.

Paralisia da mirada lateral relacionada a cavernoma da ponte / Horizontal gaze palsy due to pontine cavernous malformation

Paralisia da mirada lateral por hemorragia pontina, secundária a cavernoma, o qual apresenta prevalência estimada de 0,4% a 0,6%. O risco de sangramento de tal entidade é considerado baixo (0,1 a 3,1% ao ano). Relatamos o caso de paciente feminino, 38 anos, admitida no setor de emergência com quadro de cefaleia, vertigem, hipertensão (PA 200/120mmHg), rebaixamento do nível de consciência (Glasgow 13) e paralisia do olhar conjugado lateral à direita, com 24 horas de evolução. A tomografia de crânio revelou hemorragia pontina e a angiorressonância evidenciou a presença de cavernoma no tegmento pontino. Foi optado por tratamento conservador e a paciente evoluiu com síndrome do encarceramento (Locked-in syndrome) por piora da hemorragia e edema perilesional. Os cavernomas são malformações vasculares que podem cursar assintomáticas e passar despercebidas pelos exames de imagem até o evento hemorrágico. Apesar de raro, quando este ocorre no tronco encefálico pode apresentar alta morbimortalidade. Isso reforça a importância de se avaliar a chance de sangramento dessas lesões e instituir a melhor abordagem para cada caso. (AU)

Horizontal gaze palsy due to hemorrhage of a pontine cavernous malformation, which prevalence ranges from 0.4% to 0.6%. The risk of bleeding is considered low (0.1 to 3.1% per year). It is reported a case of a 38-year-old woman admitted to the emergency department with headache, vertigo, hypertension (200/120mmHg), decreased level of consciousness (Glasgow 13) and horizontal gaze palsy to the right side, that started suddenly 24h before admission. CT scan revealed a pontine hemorrhage and MRI showed the presence of a cavernous malformation in the pontine tegmentum. Conservative treatment was chosen and the patient developed locked-in syndrome due to worsening bleeding and perilesional edema. Cavernoma are vascular malformations that can be asymptomatic and remain undetected by imaging until the hemorrhagic event. Although rare, when bleeding occurs in the brain stem, it can cause high morbidity and mortality. This report reinforces the importance of evaluation these injuries' bleeding risk and establish the best approach for each case. (AU)

Migrainous infarction: a rare and often overlooked diagnosis

Migraine is a neurological entity and a well-known independent risk factor for cerebral infarction, which mostly afflicts the young female population. Researching focal neurological signs in this subset of the population with the diagnosis of a neurological ischemic event should always take into account the migraine as the etiology or as an associated factor. The etiology of central nervous system (CNS) ischemia is considerable. Migraine, although rare, also may be included in this vast etiological range, which is called migrainous infarction. In this setting, the diagnostic criteria required for this diagnosis is extensive. Herein, we present the case of a female adolescent who submitted to the emergency facility complaining of diplopia, dysarthria, and imbalance, which started concomitantly with a migrainous crisis with aura­a challenging clinical case that required extensive research to address all possible differential diagnoses.

Ptose miogênica na distrofia muscular oculofaríngea / Miogenic ptosis in oculopharyngeal muscular dystrophy

RESUMO Relato de caso de distrofia muscular oculofaríngea, doença genética de herança autossômica dominante e uma das causas de ptose miogênica adquirida. A paciente apresentou quadro de ptose palpebral bilateral e disfagia, achados clínicos típicos da doença, foi submetida a tratamento cirúrgico da ptose, com bom resultado estético e funcional.

ABSTRACT The authors report a case of oculopharyngeal muscular dystrophy, an autosomal dominant genetic disease, which leads to miogenic ptosis. This patient presented bilateral palpebral ptosis and dysphagia and underwent ptosis surgical treatment, with a good functional and aesthetic result.

Skew deviation e ocular tilt reaction versus paralisia do nervo troclear / Skew deviation and ocular tilt reaction versus trochlear nerve palsy

Skew deviation é o desalinhamento vertical dos olhos causado por disfunção unilateral no tronco cerebral, cerebelo ou sistema vestibular periférico, comprometendo as vias por onde transitam impulsos dos órgãos otolíticos aos núcleos oculomotores. Está frequentemente associado a torção ocular e inclinação da cabeça, compondo, assim, a ocular tilt reaction. Neste artigo os autores ressaltam aspectos históricos, fisiológicos, etiopatogênicos e clínicos que contribuem para caracterizar ambas as condições, além da diferenciação clínica com a paralisia do nervo troclear.

Skew deviation is the vertical misalignment of the eyes caused by unilateral dysfunction in the brain stem, cerebellum or peripheral vestibular system, resulting imbalance in otolith input to the oculo-motor system. It's often associated with ocular torsion and head tilt, which together comprise the ocular tilt reaction. In this article the authors emphasize the historical, physiological, etiopathogenesis and clinical aspects that contribute to characterize both conditions, and help to differentiate them clinically from trochlear nerve palsy.

Translation and validation of Convergence Insufficiency Symptom Survey (CISS) to Portuguese - psychometric results / Tradução e validação do CISS para a língua portuguesa - resultados psicométricos

Purpose: Translate and adapt the Convergence Insuficiency Symptom Survey (CISS) questionnaire to the Portuguese language and culture and assess the psychometric properties of the translated questionnaire (CISSvp). Methods: The CISS questionnaire was adapted according to the methodology recommended by some authors. The process involved two translations and back-translations performed by independent evaluators, evaluation of these versions, preparation of a synthesis version and its pre-test. The final version (CISSvp) was applied in 70 patients (21.79 ± 2.42 years) students in higher education, and at two different times, by two observers, to assess its reliability. Results: The results showed good internal consistency of the CISSvp (Cronbach's alpha - α=0.893). The test re-test revealed an average of the differences between the first and second evaluation of 0.75 points (SD ± 3.53), which indicates a minimum bias between the two administrations. The interrater reliability assessed by intraclass correlation coefficient ranged from 0.880 to 0.952, revealing that the CISSvp represents an appropriate tool for measuring the visual discomfort associated with near vision tasks with a high level of reproducibility. Conclusions: The CISS Portuguese version, showed good psychometric properties and has been sown to be applicable to the Portuguese population, to quantify the visual discomfort associated with near vision, in higher education students. .

Objetivo: Traduzir e adaptar transculturalmente o questionário Convergence Insuficiency Symptom Survey (CISS) para a língua portuguesa e avaliar as propriedades psicométricas do questionário traduzido (CISSvp). Métodos: O questionário CISS foi adaptado segundo metodologia recomendada por alguns autores. O processo envolveu duas traduções e retrotraduções realizadas por avaliadores independentes, avaliação das versões seguida da elaboração de uma versão síntese e pré-teste comentado. A versão final (CISSvp) foi aplicada em 70 indivíduos (21,79 ± 2,42 anos) estudantes do ensino superior, em dois momentos distintos e por dois observadores, para avaliar a confiabilidade do mesmo. Resultados: Os resultados evidenciaram boa consistência interna do CISSvp (alfa de Cronbach - α=0,893). O teste re-teste revelou uma media das diferenças entre a primeira e a segunda avaliação de 0,75 pontos (SD=±3,53) o que indica um viés mínimo entre as duas administrações. A confiabilidade inter-examinadores avaliada pelo coeficiente de correlação intraclasse variou de 0,880 e 0,952, revelando que o CISSvp representa uma ferramenta de medida do desconforto visual associado a tarefas em visão próxima com um nível de reprodutibilidade elevado. Conclusões: O CISS versão portuguesa apresentou boas propriedades psicométricas e demonstrou ser aplicável na população portuguesa, para quantificar a o desconforto visual associado à visão de perto, em estudantes do ensino superior. .

A Case of Ocular Myasthenia Gravis Presenting as Double Depressor Palsy

A 65-year-old man who had been experiencing diplopia in front and down gaze for 15 days visited our hospital. Hypertropia was noted in the patient's left eye, and limitation of depression was found in the adduction, primary gaze, and abduction. Brain magnetic resonance imaging showed no remarkable findings. Two weeks after the first visit, the patient complained of ptosis in the left eye. An ice test was performed and the ptosis was resolved after the test. Then, anti-acetylcholine receptor binding antibody levels were checked and found to be slightly elevated. We prescribed methylprednisolone per os 24 mg for 2 weeks, and his symptoms improved after the 2-week treatment. Five weeks after his first visit, the patient showed an ortho result in the alternate prism cover test and normal ocular movements. This may be the first case in which ocular myasthenia gravis presented as double depressor palsy, and in such cases, the possibility of ocular myasthenia gravis should be considered to rule out double depressor palsy.

Ethmoidal Sinus Mucocele as a Cause of Acquired Brown Syndrome

No abstract available.

Presentación de dos casos con desviación vertical disociada relacionada con patrón en A / Presentation of two cases of dissociated vertical deviation related to A-pattern

La desviación vertical disociada es un fenómeno bilateral, asimétrico, en el cual se presenta un movimiento de elevación, abducción y exciclotorsión durante la oclusión monocular, que generalmente es más evidente en el ojo no fijador. Cuando se asocia a patrones en A y sobreacción de ambos oblicuos superiores, se conoce como tríada que puede ser primaria o secundaria a cirugía de esotropía. Al planear la cirugía en estos casos siempre hay que tener presente la presencia de simetría y el grado de incomitancia. Las opciones quirúrgicas estarán en dependencia si es una tríada primaria no cirugía previa o si estamos ante una tríada secundaria. Presentamos dos pacientes con tríada secundaria donde, a pesar de tener el mismo diagnóstico, el planteamiento quirúrgico es diferente. Se realiza en una paciente recesión de ambos rectos laterales 7 mm con desplazamiento de la inserción hacia abajo la mitad de la inserción y miectomía oblicuo inferior derecho y en el otro caso recesión asimétrica de ambos rectos superiores 6 mm ojo derecho y 4 mm ojo izquierdo

The dissociated vertical deviation is a bilateral, asymmetric phenomenon, in which an elevation, abduction and excyclotorsion movement occurs during the monocular occlusion that is generally more evident in the non-fixing eye. When the vertical deviation is associated to A-patterns and overreaction of both superior oblique muscles, then this entitiy is known as triad that can be primary or secondary to esotropia surgery. In planning one surgery for these cases, it is always necessary to take into account the symmetry and the non-commitance grade. The surgical options will depend on whether it is a primary not prior surgery triad or a secondary triad. Here are two patients with secondary triad who, despite their similar diagnosis, the surgical plan for both were different. One patient underwent about 7 mm recession of both lateral rectus muscles with the insertion displacement downwards half of the insertion and myectomy in the right inferior oblique muscle whereas asymmetric recession of both superior rectus muscles, 6 mm for the right eye and 4 mm for the left eye, was performed in the other patient

Joubert Syndrome.

Joubert syndrome is a very rare condition seen in our country. Herein, we report a case of Joubert syndrome in a one year four months old, male baby from a consanguineous marriage presenting with delayed developmental milestone, hypotonia, abnormal respiratory pattern and nystagmus . Cranial MRI shows ‘‘Molar Tooth Sign’’.

Dynamic distance direct ophthalmoscopy, a novel technique to assess accommodation in children.

Aim: To describe and compare dynamic distance direct ophthalmoscopy (DDDO) with dynamic retinoscopy (DR) in assessment of accommodation in children. Materials and Methods: This prospective observational study had four components. Component 1: to understand the characteristic digital images of DDDO. Component 2: to compare DDDO with DR for detection of accommodative defects in children (1–16 years). Component 3: to compare DDDO with DR for the detection of completeness of pharmacologically induced cycloplegia in children (5–16 years) and Component 4: to assess which one of the two techniques was more sensitive to detect onset of cycloplegia after instillation of 1% cyclopentolate eye drops. Results: Component 1: image analysis of DDDO on two subjects (7 years and 35 years) demonstrated superior pupillary crescent that progressively disappeared with increasing accommodation. Concurrently an inferior crescent appeared that became bigger in size with increasing accommodation. Component 2: the prevalence of defects in accommodation was 3.3% (33/1000 children). Three had unilateral accommodation failure. Sensitivity of DDDO was 94%, specificity 100%, positive predictive value 100%, negative predictive value 99%, and clinical agreement (kappa) 0.97. Component 3: in the detection of completeness of pharmacologically induced cycloplegia (n=30), the sensitivity of DDDO was 94%, specificity 96%, positive predictive value 97%, negative predictive value 93% and kappa 0.9. DR had two false positives. DDDO had one false negative. Component 4: DDDO detected onset of pharmacologically induced cycloplegia 5 min earlier than DR (n=5). Conclusion: DDDO is a novel, simple, clinical and reliable method to assess accommodation in young children. This test can assess the accommodative response of both eyes simultaneously.

Tularemia and the oculoglandular syndrome of Parinaud

An 18-year-old female came to our clinic with complaints of a tender lump just under her jaw on the left side and another lump in front of her left ear, ecchymosis around the eye and some redness in the eye at the same side. After administering antibiotic therapy for two days we suspected of tularemia and referred the patient to the Infectious Diseases Department. A serum sample was taken and a fine needle aspiration biopsy was performed. The patient was diagnosed with tularemia, the oculoglandular syndrome of Parinaud. Tularemia should also be kept in mind for differential diagnosis in patients with both ocular and glandular symptoms in endemic regions like Turkey and the appropriate therapy should be initiated immediately.

Síndrome de Brown inflamatória: relato de caso / Inflammatory Brown syndrome: case report

A síndrome de Brown enquadra-se no grupo de estrabismos restritivos e é causada pela limitação de movimento do tendão do oblíquo superior através da tróclea. Caracteriza-se por ortotropia ou hipotropia na primária do olhar, limitação ou ausência de elevação em adução, frequente depressão do olho em adução com anisotropia em V e dução passiva positiva na elevação em adução. Denomina-se síndrome de Brown inflamatória, o distúrbio secundário, que tem como principais causas inflamações locais na órbita e doenças inflamatórias como artrite reumatóide e tenossinovite. Apresentamos o caso de um paciente de 44 anos, do sexo masculino, com queixa de diplopia binocular vertical à dextroversão recorrente. Diagnosticado como síndrome de Brown inflamatória sem etiologia definida, confirmada por ressonância nuclear magnética e com resolução espontânea.

Brown syndrome fits the group of restrictive strabismus and is caused by a movement limitation of the superior oblique tendon through the trochlea. It is characterized by parallelism in the primary gaze position, limitation or absence of elevation in adduction, frequent depression of the eye in adduction with anisotropy in V-pattern and positive passive duction in the elevation in adduction. It is called inflammatory Brown syndrome, a secondary disorder, which main causes are local inflammation in the orbit and inflammatory diseases such as rheumatoid arthritis and tenosynovitis. A case of a 44 year-old patient, male, complaining of binocular vertical diplopia due to recurring dextroversion is reported. It was diagnosed as inflammatory Brown syndrome of uncertain etiology, confirmed by magnetic nuclear resonance, and with spontaneous resolution.

Further diffusion tensor imaging contribution in horizontal gaze palsy and progressive scoliosis / Contribuição adicional das imagens por tensores de difusão em paralisia do olhar conjugado horizontal associada a escoliose progressiva

In two siblings with clinical diagnosis of horizontal gaze palsy associated with progressive scoliosis (HGPPS) we could demonstrate by diffusion tensor imaging: (1) An anterior displacement of the transverse pontine fibers; (2) Posterior clumping of the corticospinal, medial lemniscus and central tegmental tracts and of the medial and dorsal longitudinal fasciculi complex; (3) Absent decussation of superior cerebellar peduncle. Those findings can contribute as surrogate markers for the diagnosis.

Em dois irmãos com diagnóstico clínico de paralisia do olhar conjugado horizontal associada a escoliose progressiva, foi possível determinar através de imagens por tensores de difusão: (1) Deslocamento anterior das fibras pontinas transversas; (2) Agrupamento posterior do trato córtico-espinhal, lemnisco medial e trato tegmentar central e complexos dos fascículos longitudinais medial e dorsal; (3) Ausência da decussação dos pedúnculos cerebelares superiores. Tais achados podem contribuir como marcadores para o diagnóstico.

Desviación oblicua: diagnóstico estrabológico y alternativas de tratamiento / Skew deviation: strabismological diagnosis and treatment alternatives

Objetivo: Analizar las posibilidades de diagnóstico y tratamiento en pacientes con skew deviation (SD) o estrabismo con desviación oblicua. Material y métodos: Estudio prospectivo, observacional y longitudinal de pacientes con SD, de septiembre de 2007 a mayo de 2008. Se realizó exploración estrabológica, estudio multidisciplinario y se evaluaron alternativas terapéuticas. Resultados: Se estudiaron 10 pacientes con SD. Edad 11.5 + 13.6 años. Los diagnósticos neurológicos fueron esclerosis múltiple, malformación arteriovenosa, epilepsia, hidrocefalia, encefalopatía isquémica, atrofia cortical, hipoplasia de cuerpo calloso y hemorragia talámica. Se encontró retraso psicomotor en 80 %, además se asoció a apraxia de Cogan, síndrome de Parinaud, nistagmo en see-saw, síndrome de Foville y hemiplejía. Los estrabismos relacionados fueron exotropía en cinco, endotropía en tres, hipertropía en dos, desviación vertical disociada en uno. Se encontró lesión a II, III y VII nervios craneales. Conclusiones: El estudio estrabológico adecuado permite un mejor diagnóstico de la lesión en SD y con ello vigilar la evolución neurológica del paciente. El tratamiento debe incluir la rehabilitación óptica y el manejo del estrabismo, principalmente con toxina botulínica.

BACKGROUND: We undertook this study to analyze diagnostic and treatment alternatives in patients with skew deviation (SD). METHODS: This is a prospective, observational and longitudinal study of patients with SD. The study took place in a third-level medical center during the period from September 2007 to May 2008. Strabismological exploration, multidisciplinary diagnosis and treatment alternatives were analyzed. RESULTS: Ten patients presenting SD were studied. Diagnoses were multiple sclerosis, arteriovenous malformation, epilepsy, hydrocephalus, ischemic encephalopathy, cortical atrophy, hypoplasia of corpus callosum and thalamic hemorrhage. Psychomotor retardation was present in 80%. Other diagnoses were Cogan apraxia, Parinaud syndrome, see-saw nystagmus, Foville syndrome, and hemiplegic alterations. Related strabismuses were exotropia (5), esotropia (3), hypertropia (2), and dissociated vertical deviation (1). Lesions of II, III and VII cranial nerves were found. CONCLUSIONS: Complete strabological study allows a better diagnosis of the lesion and consequently relapsing disease in order to achieve a better treatment according to each patient. Optical rehabilitation and botulinum applications are especially indicated.